Afectación intracraneal en la neurosarcoidosis: descripción de cuatro casos como manifestación inicial de la enfermedad

Introduction. The incidence of sarcoidosis in our country is one of the lowest in Europe. Neurosarcoidosis affects only 5% on patients with sarcoidosis. Clinical cases. We describe four patients in which neurologic disfunction was the presenting finding. Initial neurological symptoms include status epilepticus, headache, fever and nerve cranial palsies. MRI showed a spectrum of protean central nervous abnormalities: 1. Hypotalamic infiltrating lesion; 2. Brain parenchyme enhanced masses; 3. Leptomeningeal enhancement, and 4. Focal white-matter lesions. Thoracic CT scan, bronchoscopy, Gallium scintigraphy and pulmonary biopsy yielded to diagnosis in three patients. Biopsy of the meninges was required in one patient because systemic involvement was not found. Histological examination of an intracranial was was also performed in another patient to rule out tumoral lesions. ACE in serum was normal in all patients. CSF ACE was determine in only one patient and was also normal. Three patients started treatment with corticosteroids but one of them required adjuvant treatment with immunosuppressor. Cranial nerve palsy resolved spontaneously in the last patient. Conclusions. Clinical and radiological polymorphism explained the delay before diagnosis and the problems in ruling out other disease. diagnosis and follow up treatment.